Paediatrics Flashcards
CCCC
Punched out lytic skull lesions with bevelled edges?
Eosinophilic granuloma
Ground glass appearance lytic skull lesion ?
Fibrous dysplasia
Addition: Cortical expansion and well-defined sclerotic margins
Well-defined lucencies paired in the posterior para-sagittal location?
Parietal Foramina
Monoventricle, thalami and basal ganglia are fused?
Alobar Holoprosencephaly
Addition: Most severe form.
**No falx or corpus callosum
Complete absence of cleavage with “pancake” of anterior cerebral tissue, crescent-shaped anterior monoventricle communicating with large dorsal cyst,** fused thalami**
Absent septum pellucidum ?
Lobar Holoprosencephaly
Mildest from of holoprosencephaly
Cleavage is apparently back to front (opposite the corpus callosum ) so posterior fossa is normal in mildest form
Interhemispheric fissure & falx are mostly formed with partial nonseparation of frontal lobes
Partially formed falx, rudimentary third ventricle, fused at the thalamus. posterior brain is normal?
Semilobar Holoprosencephaly
Absent frontal lobe cleavage with parietooccipital lobe separation
Soft tissue neck lump anterior border of SCM and posterior to the submandibular gland?
2nd Branchial cleft cyst
Nb- can extend between the carotid bifurcation but doesn’t splay the bifurcation ie carotid body tumour (Chemoductoma) (age group 50-60s)
DDx Necrotic level 2 node
-older child/adult
-Thyroid cancer or HPV related nasopharyngeal
Soft tissue lump at the ‘peri-parotid’ region?
1st Branchial cleft cyst
Cystic mass near pinna & EAC or extending from EAC to angle of mandible
Soft tissue lump at the base of the SCM?
3rd Branchial cleft cyst
Multilocular fluid filled mass with internal septations at the the posterior triangle?
Cystic hygroma
Nb - Associated with turners and hydrops/downs
T2 bright. Doesnt enhance. No internal flow.
Difference and similarities between Epidermoid cyst and arachnoid cyst
Both - CSF density on CT, can occupy CPA, homogeneous.
Epidermoid - Restricts diffusion. Hyperintensity on FLAIR
Complete opacification of the maxillary sinus, peripheral enhancemen, expanded sinus with smooth remodeling of walls?
Mucocoele
Nb - Ostium of given sinus is occluded thus complete opacification.
*- Doesn’t extend beyond the cavity. *
- **Expands cavity and can cause bone thinning. **
Associated with* CF and prior trauma*
Peripheral enhancment allows differentiate from neoplasm
Low attenuation mass in the sinus that expands the cavity and extends into the meatus ?
Antrochonal polyp/Solitary Sinonasal Polyp
*Nb - T2 bright and also peripheral enhancement. *
Typically doesn’t completely opacify the sinus
Solitary ‘dumbell shaped-‘ polypoid mass fills maxillary antrum, then spills through enlarged maxillary ostium and infundibulum or accessory ostium into nasal cavity
Seizures, atrophy of hippocampus and high T2/FLAIR signal?
Mesial temporal sclerosis
Difference between Limbic encephalitis and HSV in paediatric setting?
Limbic-
Paraneoplastic (bronchial small cell) or autoimmune.
Psychotic features.
No haemorrhage on imaging
HSV -
Can have haemorrhage. Cytotoxic oedema and mass effect.
- HSV -1 . (Adults HSV-2)
- DWI restriction
- Bilateral but asymmetric with late hemorrhage
- Basal ganglia usually spared
Sperate it from Japanese encephalitis = doesnt spare the basal ganglia/thalamus.
Intraventricular lobulated mass in trigone of lateral ventricle. Smooth, large and small foci of calcification on CT. Lateral ventricle is dilated?
Choroid plexus papilloma
Hyperdense on CT.
Iso on T1 and hyperintense on T2.
**Intense homogenous enhancement. **
Nb in adults - occur predominantly in 4th ventricle
Mass in the inferior 4th ventricle of child with hypointensity on T1 and foci of high T2 signal ?
Ependymoma
4th ventricle mass (± indistinct interface with floor of 4th ventricle)
Fine, stippled Ca⁺⁺ common (50%)
± cysts, hemorrhage
Hydrocephalus common
Hetergenous Signal as Ca2+/Blood products
- overall T1 iso/hypointense and T2 hyerpintense
Anterolateral extension through recess(es) into CPA cistern
Posteroinferior extension through foramen of Magendie into cisterna magna
Enlargement of the SCM in the neonatal period ?
Fibromatosis coli
‘Two heads of the SCM’
Erosive soft tissue mass in the middle ear ?
Cholesteatoma
Nb - its termed ‘Congenital’ if less than < 5yrs.
Isointense T1 and hyperintense T2
Congenital cholesteatoma are less destructive.
Non- erosive soft tissue mass in the middle ear ?
Choleterol granuloma
Nb - Cystic strucutres with Blood and cholesterol =** High T1 and T2.**
If located in petrous apex can be aggressive
Most common cause of premature suture closure?
Sagittal
Scaphocephaly - ‘scapho’ latin for boat - LONG and NARROW
Trigonocephaly = **POINTED forehead = closure of metopic **suture, eyes close together
Brachycephaly - Coronal. = SHORT and WIDENDED
Unilateral more common. Harlequins eye
Turricephaly (bilateral) or Plagiocepahly (unilateral) - Lambdoid
Cloverleaf - all
Early closure of which suture causes elevation of the superolateral corner of the orbit?
Coronal
Harlequin eye / Brachycephaly
What brain tumour is found in Tuberous Sclerosis?
Subependymal giant cell astrocytoma (SEGA)
aka Intraventricular astrocytoma of tuberous sclerosis complex (TSC
Enlarging, enhancing foramen of Monro mass in patient with TSC
Tuberous sclerosis is triad of
-facial angiofibromas
-seizures
-mental retardation
Cortical/subcortical TUBERS
- expand overlying gyri, low on CT,
- can have cystic and Ca2+ transformation.
DDX - TORCH namely toxo and CMV can cause periventricular Ca2+
What are the GU/GI manifestations of Tuberous sclerosis?
Renal cysts,
Renal AMLs
splenic adenoma
Hamartomatous rectal polyps
What are the GI/GU manifestations of VHL?
RCC
Renal and pancreatic cysts
Phaeochromocytoma
What are the GI/GU manifestations of NF-1
Hirschsprungs
Carcinoid
Renal artery stenosis
Nb none with NF-2
Highly vascular mass in nasopharynx eroding the medial ptyergoid plates and arising from sphenopalatine fossa?
Juvenile angiofibroma
Supplied by the internal maxillary artery. Embolise it.
Pineal mass in boy with precocious puberty?
Germinoma
Pineal mass with Central localized Ca⁺⁺ & bithalamic extension
Nb Germinoma and Pineoblastoma difficult to differentiate on imaging.
Can be Suprasellar mass with diabetes insipidus (DI)
Child with large, heterogeneous hyperdense pineal mass with peripheral Ca⁺⁺
Pineoblastoma
**Scattered (“exploded”) Ca⁺⁺ rather **than localized (“engulfed”) Ca⁺⁺ in germinoma
Differentiate a medulloblastoma from a pilocytic astrocytoma in the posterior cranial fossa
Medulloblastoma
Midline 4th ventricular mass in 1st decade of life
Hyperattenuating (CT)
diffusion restricting (MR)
PCA -
Cystic cerebellar mass with enhancing mural nodule
Enlarged optic nerve/chiasm/tract with variable enhancement
Hypo/Isodense. Ca2+ often
Distinguishing features if ADEM?
Post viral. Young adults or children
Demyelination with bilateral asymmetrical T2/FLAIR hyperintensities (confluent or punctuate).
Thalamic involvement and cranial nerves (not typcial MS)
Dramatic response with steorids
What is Hurst disease?
Fulminant haemorrhagic version of ADEM
Rapidly progressive ataxia, ophthalmoplegia, dystonia, Lactic acidosis.
High T2 putamen, caudate periaqueductal grey matter?
Leigh Disease
Symmetric “speckled” lesions in basal ganglia (BG)
**L for Lactic acidosis **
What is a Pott puffy tumour?
during acute sinusitis, bony erosion and subperiosteal abscess formation. Causes forehead swelling and can have intracranial extension.
Chondrocalcinosis, premature OA changes and dark pigmentation in skin, particularly the ears?
Alkaptonuria / oschronosis
Nb Can be diagnosed in infancy - nappies stained black from urine.
AR condition. Excessive levels of homogentisic acid (HGA), affinity to accumulate in connective tissues.
Calcification and destruction intervertebral discs.
History of epilepsy, young, temporal lobe lesion, multi-cystic lesion?
Dysembryoplastic neuroepithelia tumours (DNET)
**Nb - Multiple cysts in the lesion ‘bubbly’ **.
Lack of oedema or enhancement is typical
DDX for a temporal lobe lesion and epilepsy -
—Ganglioglioma (not bubbly, can be cystic and usually enhance)
Bilateral painless parotid swellings with mixed cystic and solid lesions on US. Main DDX?
Sjorgens
HIV
Sarcoidosis (rare)
Mumps
Differentiate -
–HIV typically has cervical lymphadenopathy, not painful amd NO calcifcations.
–Sjogren’s Middle-aged female demographic &/or intraglandular calcifications
— mumps are painful
Baby, developmental delay, normal head size but diffuse white matter abnormalities with a stripey ‘trigoid’ appearance
Metachromatic Leukodystrophy
**Normal head size **
Most common leukodystrophy. Trigoid or butterfly appearance.
Spares subcortical u fibres
Macrocephaly in child less than 12 months, diffuse bilateral WM changes and subcortical u fibres, elevated NAA peak levels?
Canavans disease
Macrocephaly with diffuse ↑ white matter (WM) T2 and DWI signal, and ↑ N-acetyl aspartate (NAA) in MRS
**Subcortical u fibres **
Macrocephaly in child less than 12 months , frontal white matter Chages?
Alexanders disease
Symmetric, ↑ T2-signal bifrontal white matter (WM), spare u fibres
5-10 year old, WM changes that in the occipital lobes and splenium of CC, that progress frontal. Elevated serum VLCFA ?
Adrenoleukodystrophy
Baby under 6 months old, RUQ mass, cardiomegaly ?
Haemangioendothelioma (aka Infantile hepatic haemangiomas (IHH))
**Fine calcifications. **
Often found on foetal antenatal scans!!!
Can be solitary or multiple
Nb - behave like giant haemangioma on imaging -
Heterogenous on US, peripheral enhancement with gradual filling in on CT, flow voids on MRI.
can cause significant R-L heart shunting.
AFP + Endothelial growth factor is elevated.
Thrombocytopenia due to platelet trapping
Nb - not like adult epitheliod haemangioendothelioma
Painless abdominal mass, 6 year old child. US - large well defined solid heterogenous liver mass with raised AFP?
Hepatoblastoma
Coarse calcification
Precocious puberty is highly specific but rare.
Most common malignant hepatic tumour of childhood.
Young adolescents = HCC
Associated with* Beckwith Wiedemann syndrome. *
Can invade the vessels
Large abdominal mass in neonate, with respiratory compromise. Multicystic and enhancing septations.
Mesenchymal hamartoma
Benign tumour. Sheer size can cause diaphragms splinting.
No Ca2+ or haemmorhage.
May grow rapidly, leading to compartment syndrome, respiratory distress, rarely heart failure
AFP may be moderately elevated
Imaging: Multiloculated large cystic mass in 70%;
What is the best test to diagnosis Meckel diverticulum?
Technetium - Pertechnetate studies
Syndrome linked with Chromophobic RCC?
Birt-Hogg-Dube
Also pulmonary cysts (increased risk of spontaneous pneumothorax) and fibrofolliculomas (benign fibrous tumours related to the hair follicles).
Syndrome’s linked with Wilms tumours?
**Beckwith-Wiedmnann (3 montly US for screening) **
DRASH
WAGR
Neonatal jaundice, normal US and 6 and 24 hours HIDA scan show no excretion?
Biliary atresia
US - sometimes shows echogenic ‘triangular cord’ structure in the porta hepatis which is pathogonomic
DDx - Neonatal hepatitis - 6 or 24 hours HIDA will show bowel uptake
Nb
Hepatic activity after 5 minutes and no bowel activity after 6 and 24 hours
Main causes for Neonatal distal intestinal obstruction with microcolon?
A. Meconium Ileus
Meconium impacted at the terminal ileum. Linked to CF.
On WSCE - outlines meconium pellets obstructing terminal ileum.
B. Ileal atresia
On WSCE - Blind end of contrast column in ileum = Ileal atresia
C. Total colonic Hirschsprung disease
Main causes of neonatal distal intestinal obstruction with small distal /left colon?
A. Meconium plug syndrome (neonatal small left colon)
Nonpathological transient functional obstruction at the proximal descending colon**.
+/- if water souble contrast isnt therapeutic then - Rectal biopsy to exclude long segment HD
B. Colonic atresia
WSCE - blind ending small caliber distal colon (can be more proximal)
C. Hirschsprung’s disease
WSCE - Classically short rectosigmoid segment = small calibre rectum and dilated sigmoid (rectosigmoid ration <1).
Long segment - Transition zone from the small distal colon to the dilated proximal colon
**suction biopsy below this transition point.
Older child with distal intestinal obstruction or right iliac fossa pain/mass and normal appendix. Bilateral upper zone bronchiectasis ?
Distal intestinal obstruction syndrome
Secondary to CF
Older child meconium ileus equivalent